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Gene Therapy for Inborn Errors of Immunity
The Journal of Allergy and Clinical Immunology: In Practice, 2023In the early 1990s, gene therapy (GT) entered the clinical arena as an alternative to hematopoietic stem cell transplantation for forms of inborn errors of immunity (IEIs) that are not medically manageable because of their severity. In principle, the use of gene-corrected autologous hematopoietic stem cells presents several advantages over ...
Tiphaine Arlabosse +2 more
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Thymic inborn errors of immunity
Journal of Allergy and Clinical ImmunologyThe thymus is crucial for optimal T-cell development by facilitating the generation and selection of a diverse repertoire of T cells that can recognize foreign antigens while promoting tolerance to self-antigens. A number of inborn errors of immunity causing complete or partial defects in thymic development (athymia) and/or impaired thymic function ...
Francesca, Pala +2 more
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COVID-19 and Inborn Errors of Immunity
Physiology, 2022Inborn errors of immunity (IEI) are a heterogeneous group of disorders affecting immune host defense and immunoregulation. Considering the predisposition to develop severe and chronic infections, it is crucial to understand the clinical evolution of COVID-19 in IEI patients.
Ottavia M. Delmonte +2 more
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Otitis Media and Inborn Errors of Immunity
Current Allergy and Asthma Reports, 2020The aim of this review is as follows: (1) to present the role of otitis as a warning sign for inborn errors of immunity (IEI), (2) to establish which patients presenting otitis should be investigated for IEI, (3) to review data about main IEI associated with otitis-prone patients.Otitis media is a very common infection in general population.
Maine L D, Bardou +2 more
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Proteasome disorders and inborn errors of immunity
Immunological Reviews, 2023SummaryInborn errors of immunity (IEI) or primary immune deficiencies (PIDD) are caused by variants in genes encoding for molecules that are relevant to the innate or adaptive immune response. To date, defects in more than 450 different genes have been identified as causes of IEI, causing a constellation of heterogeneous clinical manifestations ranging
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Viral infections and inborn errors of immunity
Current Opinion in Infectious DiseasesPurpose of review The purpose of this focused review is to discuss unusual presentations of viral infections in the context of specific inborn errors of immunity. We will discuss hyper immunoglobulin E (IgE) syndromes, epidermodysplasia verruciformis, and X-linked agammaglobulinemia as examples of inborn errors of immunity ...
Anne, Ewing, Rebecca Pellett, Madan
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The lung in inborn errors of immunity
Pediatric PulmonologyAbstractThe lungs are integral to immune defense, and inborn errors of immunity (IEI) often manifest as lung disease. Lung complications of IEI can involve the airways, alveolar spaces, interstitium, vasculature, and pleura. Accurate identification of these lung disease patterns requires a thorough clinical history, physical examination, and high ...
Sonia Restrepo‐Gualteros +2 more
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[Genetics of inborn errors of immunity].
Zeitschrift fur Rheumatologie, 2022In the last 10 years there has been enormous progress in the field of inborn errors of immunity (IEI). The number of newly discovered diseases is growing exponentially, including not only rare but also frequent genetic defects. The spectrum of clinical phenotypes ascribed to IEI is also rapidly expanding.
Faranaz, Atschekzei, Gesine, Schürmann
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[Autoinflammatory syndromes: inborn errors of natural immunity].
Recenti progressi in medicina, 2007The notion of autoinflammatory diseases delineates a heterogenous group of genetic pathologies characterized by spontaneous periodic systemic inflammation in the absence of infectious or autoimmune causes. The general hypothesis is that the innate immune response in these patients is wrongly tuned, being either too sensitive to minor stimuli or turned ...
COLINA, Matteo +6 more
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